6 th EUROPEAN SYMPOSIUM ON RARE ANAEMIAS 1 st Dutch-Belgian meeting for patients and health professionals

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1 6 th EUROPEAN SYMPOSIUM ON RARE ANAEMIAS 1 st Dutch-Belgian meeting for patients and health professionals

2 Dr. Marjon H. Cnossen, paediatric haematologist Erasmus MC-Sophia Children s Hospital, Rotterdam, the Netherlands Disclosures Company name Novartis Nordic Pharma Research support X X Consultant Stockholder Speakers bureau Advisory board Other

3 This talk is applicable for: Thalassemia s Sickle cell disease Membrane disorders (e.g. spherocytosis) Enzym defects (e.g. PKD, G6PD) PNH Other forms of haemolytic disease Definite X X X X X Probable

4 P

5 Paediatric haematologist Often congenital, chronic diseases Family doctor (patient, parents, siblings) Mild to severe complications, hospital admissions, therapeutic interventions

6 Neonatal screening programmes

7 Frequent Hospital Admissions

8 Organ damage in sickle cell disease Retinopathy Sec. haemochromatosis Gall stones Bone necrosis Cerebral infarctions Cardiomyopathy Pulmonary Hypertension Asplenia Nephropathy Priapism Ulcera Spleen: removes bacteria from blood stream, especially capsultated type

9 Intensive follow up organ damage

Prophylactic antibiotics Preventive care: education, follow up organ damage (TCD) Early interventions and

10 Sickle cell disease (SCD): paediatric morbidity, mortality Development into chronic disease Early diagnosis: neonatal screening programmes Comprehensive care teams: multidisciplinary, dedicated care Prophylactic antibiotics Preventive care: education, follow up organ damage (TCD) Early interventions and treatment symptoms, complications (Hydroxycarbamide, chronic transfusions) Haematological stem cell transplantation (HSCT)

11 Transition of care Definition Purposeful, planned process with goal of providing continuity of care, preparing young adults for greater independence. Goal: to maximize lifelong functioning and potential through adequate health services. Multifaceted, vibrant process, initiated with child-parent focus and ending with adult-focused health care orientation.

12 Importance: Transition of care Impact both patient and parents Increased rate emergency visits, morbidity, early mortality SCD: Additional impact neurocognitive impairments Other disease specific problems

13 Core elements (gottransition.org) Health care transition policy Identification of transitioning youth Assessing, tracking transition readiness Adressing needs with youth and family Paediatrician, internist working together to safeguard process Contact after transition for evaluation

14 Child-centered Care A different world

15 Children s book: Maisa and the Moon

16 Adult-oriented Care

17 What is important? Recommendations Transition planning (<12 years), written transition plan Neurocognitive testing Questionnaires (generic, disease specific) Transition team Transfer of care Bryant et al J Ped Onc;

18 What is known? Questionnaires Transition Readiness Assessment Questionnaire (autonomy: Do you - ) Adolescent Assessment Preparation Transition (autonomy: Did you - ) Sickle Cell Self Efficacy Scale ( How sure are you - ) Quality of life (PEDQoL, TAPSQoL) TRAQ: D. Wood, et al. 2014; Sawicki et al. 2015; Clay et al. 2008;

19 Anxiety and depression, HRQoL in SCD Majority studies: higher risk depressive symptoms, lower HRQoL Associated with disease severity, lack of parental/social support Behavioural changes associated with hospital admissions (internalization, aggression, sleeping disorders) Anxiety, depression associated: pain, poor treatment adherence Treatment/ training Cognitive behaviour therapy (pain, coping strategies, depression, adherence)

unfortunately not KLIK Questionnaires (generic,

22 Dutch Transition Transition planning (<10 years), Individual Transition Plan Neurocognitive testing: unfortunately not KLIK Questionnaires (generic, disease specific), SCSES Transition team, Transfer of care

23 Transitional process Development with regard to specific care domains 1. Knowledge of the disease 2. Adherence treatment : autonomy, self efficacy, quality of life 3. Disease inheritance 4. Emotional development: anxiety, depression Individual transition plan (focus on my - disease, health, autonomy, role at home, education and future, finances and mobility, free time and friends, relationships and sexuality?)

24 Transitional protocol: Erasmus MC-Sophia

25 Cyberpoli (Stichting artsen voor kinderen) Lex Winkler

26 Endemic other parts of the world

27 Education in all languages Mobile Chinese Tolk

29 Provoking factors for sickle cell crisis

KLIK and self efficacy QoL questionnaires www.hetklikt.

30 KLIK and self efficacy QoL questionnaires AMC-Emma Children s Hospital, Amsterdam; Prof. Dr. M. Grotenhuis

31 Group consultations (TEAM study) Motivational interviewing

32 Self empowerment

33 Hemoglobinopathies: Inheritance

34 Sickle cell- and Thalassemia team

Dr. C. Catsman Neurology (pediatric) eurologist Dr. L. van den Toorn, H. Tiddens Pulmonology (pediatric) ulmonologist Sikkelcel team: Team effort! Dr. Dr. A.W. Rijneveld, F.N. Croles, M.J. Claessen M.

35 Dr. C. Catsman Neurology (pediatric) eurologist Dr. L. van den Toorn, H. Tiddens Pulmonology (pediatric) ulmonologist Sikkelcel team: Team effort! Dr. Dr. A.W. Rijneveld, F.N. Croles, M.J. Claessen M.H. Cnossen, C.H. van Ommen, E.J. Huisman, P. de Pagter Hematology Pediatric Hematology internist hematologist pediatric hematologist Mw. A.Goncalvez da Silva, J. van Onna Hematology sickle cell nurse (adults) Mw. S. Teuben Pediatric Hematology sickle cell nurse (children) Mw. R. Lachminarainsingh, F. van Veelen Social Service Department social worker (adults, children) F. Petrij, A.M.S. Joosten Clinical Genetics clinical geneticist Dr. F.J. Smiers, A. Lankester Hematology-immunology, LUMC pediatric hematologist Diagnostics and scientific research Dr. K. van Lom Hematology, Erasmus MC Head Diagnostic Laboratory Dr. C. Harteveld Hemoglobinopathy Laboratory, LUMC Head Diagnostic Laboratory Prof. Dr. J. Philipsen Cell Biology, Erasmus MC Head of Cell Biology Laboratory Dr. M.J. Madderom Psychiatry, Erasmus MC Adherence research Dedicated subspecialists Dr. P.K. Bos Orthopedics (pediatric) orthopedic surgeon Prof. Dr. J. Roos, L. Koopman Cardiology (pediatric) cardiologist Dr. J.J. Duvekot, A. Mulders, E.A.P. Steegers, W. Visser Obstetrics and Gynaecology gynaecologist Dr. R.W.A.M. Kuijpers Ophthalmology ophthalmologist Dr. L. Visser, R. Tenbrinck Anesthesiology anesthesiologist

36 Thank you for your attention!

Partnership between sickle cell nurse and families in outpatient clinic

Partnership between sickle cell nurse and families in outpatient clinic Ngalula Mujinga Malou Nurse (sickle cell disease) University Children Hospital Queen Fabiola (HUDERF)(Belgium) 6 th EUROPEAN SYMPOSIUM